Abstract

A 70-year old man developed achromatopsia with bilateral loss of superior visual fields and an inability to recognize familiar faces (prosopagnosia). Ophthalmologic examination results were normal. Visual acuity was 20/25 in either eye. Computerized axial tomography of the brain revealed infarction of the inferior aspect of the temporal occipital cortex in both hemispheres. The patient’s complaint that objects appeared only in shades of gray was supported by large errors made throughout the spectrum on the Farnsworth-Munsell 100 hue test and by matches over the entire red/green range on the Nagel anomaloscope. Although absolute scotopic and photopic thresholds were unremarkable, the increment thresholds to a 482-nm test on a red background increased monotonically as if the ∏1 mechanism were absent. In addition, the spectral sensitivity to large test flashes on an intense red background peaked in the middle rather than in the short-wave portion of the spectrum, as is normally found. We speculate that the chromatic channel is compromised. The patient’s residual vision is mediated by a luminance channel that is subserved by the middle and long—but not the short—wave cone mechanisms.

© 1980 Optical Society of America

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References

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  1. C. C. Meadows, “Disturbed perception of colours associated with localized cerebral lesion,” Brain 97, 615–632 (1974).
    [Crossref] [PubMed]
  2. A. L. Pearlman, J. Birch, and J. C. Meadows, “Cerebral color blindness: An acquired defect in hue discrimination,” Ann. Neurol. 5, 253–261 (1979).
    [Crossref] [PubMed]
  3. R. S. L. Young, G. A. Fishman, and F. Chen, “Traumatically acquired color vision defect,” Invest. Ophthalmol. 19, 545–549 (1980).
  4. J. Birch, “A case of acquired tritanopia,” Mod. Probl. Ophthalmol. 17, 325–330 (1976).
  5. A. L. Pearlman, “Anatomy and physiology of central visual pathways,” in Adler’s Physiology of the Eye, edited by R. A. Moses (Mosby, St. Louis, 1975).
  6. W. S. Stiles, “The directional sensitivity of the retina and the spectral sensitivity of the rods and cones,” Proc. R. Soc. Biol. 127, 64–105 (1939).
    [Crossref]
  7. W. S. Stiles, “Separation of the “blue” and “green” mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. Biol. 133, 418–434 (1946).
    [Crossref]
  8. E. N. Pugh, “The nature of π1, color mechanism of Stiles,” J. Physiol. 257, 713–747 (1976).
  9. R. S. L. Young and G. A. Fishman, “A patient with cortical colorblindness,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.
  10. S. M. Zeki, “Uniformity and diversity of structure and function in rhesus monkey prestriate visual cortex,” J. Physiol. 277, 273–290 (1978).
  11. Y. Hsai and C. A. Graham, “Spectral luminosity curves for protanopes, deuteranopes, and normal subjects,” Proc. Natl. Acad. Sci. 43, 1011–1019 (1957).
    [Crossref]
  12. P. Whittle, “The brightness of coloured flashes on backgrounds of various colors and luminances,” Vision Res. 13, 621–638 (1973).
    [Crossref] [PubMed]
  13. R. L. De Valois and K. K. De Valois, “Spatial processing of color and luminance information,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.

1980 (1)

R. S. L. Young, G. A. Fishman, and F. Chen, “Traumatically acquired color vision defect,” Invest. Ophthalmol. 19, 545–549 (1980).

1979 (1)

A. L. Pearlman, J. Birch, and J. C. Meadows, “Cerebral color blindness: An acquired defect in hue discrimination,” Ann. Neurol. 5, 253–261 (1979).
[Crossref] [PubMed]

1978 (1)

S. M. Zeki, “Uniformity and diversity of structure and function in rhesus monkey prestriate visual cortex,” J. Physiol. 277, 273–290 (1978).

1976 (2)

E. N. Pugh, “The nature of π1, color mechanism of Stiles,” J. Physiol. 257, 713–747 (1976).

J. Birch, “A case of acquired tritanopia,” Mod. Probl. Ophthalmol. 17, 325–330 (1976).

1974 (1)

C. C. Meadows, “Disturbed perception of colours associated with localized cerebral lesion,” Brain 97, 615–632 (1974).
[Crossref] [PubMed]

1973 (1)

P. Whittle, “The brightness of coloured flashes on backgrounds of various colors and luminances,” Vision Res. 13, 621–638 (1973).
[Crossref] [PubMed]

1957 (1)

Y. Hsai and C. A. Graham, “Spectral luminosity curves for protanopes, deuteranopes, and normal subjects,” Proc. Natl. Acad. Sci. 43, 1011–1019 (1957).
[Crossref]

1946 (1)

W. S. Stiles, “Separation of the “blue” and “green” mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. Biol. 133, 418–434 (1946).
[Crossref]

1939 (1)

W. S. Stiles, “The directional sensitivity of the retina and the spectral sensitivity of the rods and cones,” Proc. R. Soc. Biol. 127, 64–105 (1939).
[Crossref]

Birch, J.

A. L. Pearlman, J. Birch, and J. C. Meadows, “Cerebral color blindness: An acquired defect in hue discrimination,” Ann. Neurol. 5, 253–261 (1979).
[Crossref] [PubMed]

J. Birch, “A case of acquired tritanopia,” Mod. Probl. Ophthalmol. 17, 325–330 (1976).

Chen, F.

R. S. L. Young, G. A. Fishman, and F. Chen, “Traumatically acquired color vision defect,” Invest. Ophthalmol. 19, 545–549 (1980).

De Valois, K. K.

R. L. De Valois and K. K. De Valois, “Spatial processing of color and luminance information,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.

De Valois, R. L.

R. L. De Valois and K. K. De Valois, “Spatial processing of color and luminance information,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.

Fishman, G. A.

R. S. L. Young, G. A. Fishman, and F. Chen, “Traumatically acquired color vision defect,” Invest. Ophthalmol. 19, 545–549 (1980).

R. S. L. Young and G. A. Fishman, “A patient with cortical colorblindness,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.

Graham, C. A.

Y. Hsai and C. A. Graham, “Spectral luminosity curves for protanopes, deuteranopes, and normal subjects,” Proc. Natl. Acad. Sci. 43, 1011–1019 (1957).
[Crossref]

Hsai, Y.

Y. Hsai and C. A. Graham, “Spectral luminosity curves for protanopes, deuteranopes, and normal subjects,” Proc. Natl. Acad. Sci. 43, 1011–1019 (1957).
[Crossref]

Meadows, C. C.

C. C. Meadows, “Disturbed perception of colours associated with localized cerebral lesion,” Brain 97, 615–632 (1974).
[Crossref] [PubMed]

Meadows, J. C.

A. L. Pearlman, J. Birch, and J. C. Meadows, “Cerebral color blindness: An acquired defect in hue discrimination,” Ann. Neurol. 5, 253–261 (1979).
[Crossref] [PubMed]

Pearlman, A. L.

A. L. Pearlman, J. Birch, and J. C. Meadows, “Cerebral color blindness: An acquired defect in hue discrimination,” Ann. Neurol. 5, 253–261 (1979).
[Crossref] [PubMed]

A. L. Pearlman, “Anatomy and physiology of central visual pathways,” in Adler’s Physiology of the Eye, edited by R. A. Moses (Mosby, St. Louis, 1975).

Pugh, E. N.

E. N. Pugh, “The nature of π1, color mechanism of Stiles,” J. Physiol. 257, 713–747 (1976).

Stiles, W. S.

W. S. Stiles, “Separation of the “blue” and “green” mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. Biol. 133, 418–434 (1946).
[Crossref]

W. S. Stiles, “The directional sensitivity of the retina and the spectral sensitivity of the rods and cones,” Proc. R. Soc. Biol. 127, 64–105 (1939).
[Crossref]

Whittle, P.

P. Whittle, “The brightness of coloured flashes on backgrounds of various colors and luminances,” Vision Res. 13, 621–638 (1973).
[Crossref] [PubMed]

Young, R. S. L.

R. S. L. Young, G. A. Fishman, and F. Chen, “Traumatically acquired color vision defect,” Invest. Ophthalmol. 19, 545–549 (1980).

R. S. L. Young and G. A. Fishman, “A patient with cortical colorblindness,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.

Zeki, S. M.

S. M. Zeki, “Uniformity and diversity of structure and function in rhesus monkey prestriate visual cortex,” J. Physiol. 277, 273–290 (1978).

Ann. Neurol. (1)

A. L. Pearlman, J. Birch, and J. C. Meadows, “Cerebral color blindness: An acquired defect in hue discrimination,” Ann. Neurol. 5, 253–261 (1979).
[Crossref] [PubMed]

Brain (1)

C. C. Meadows, “Disturbed perception of colours associated with localized cerebral lesion,” Brain 97, 615–632 (1974).
[Crossref] [PubMed]

Invest. Ophthalmol. (1)

R. S. L. Young, G. A. Fishman, and F. Chen, “Traumatically acquired color vision defect,” Invest. Ophthalmol. 19, 545–549 (1980).

J. Physiol. (2)

E. N. Pugh, “The nature of π1, color mechanism of Stiles,” J. Physiol. 257, 713–747 (1976).

S. M. Zeki, “Uniformity and diversity of structure and function in rhesus monkey prestriate visual cortex,” J. Physiol. 277, 273–290 (1978).

Mod. Probl. Ophthalmol. (1)

J. Birch, “A case of acquired tritanopia,” Mod. Probl. Ophthalmol. 17, 325–330 (1976).

Proc. Natl. Acad. Sci. (1)

Y. Hsai and C. A. Graham, “Spectral luminosity curves for protanopes, deuteranopes, and normal subjects,” Proc. Natl. Acad. Sci. 43, 1011–1019 (1957).
[Crossref]

Proc. R. Soc. Biol. (2)

W. S. Stiles, “The directional sensitivity of the retina and the spectral sensitivity of the rods and cones,” Proc. R. Soc. Biol. 127, 64–105 (1939).
[Crossref]

W. S. Stiles, “Separation of the “blue” and “green” mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. Biol. 133, 418–434 (1946).
[Crossref]

Vision Res. (1)

P. Whittle, “The brightness of coloured flashes on backgrounds of various colors and luminances,” Vision Res. 13, 621–638 (1973).
[Crossref] [PubMed]

Other (3)

R. L. De Valois and K. K. De Valois, “Spatial processing of color and luminance information,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.

R. S. L. Young and G. A. Fishman, “A patient with cortical colorblindness,” presented at the 1979 Optical Society of America meeting in Rochester, New York, 1979.

A. L. Pearlman, “Anatomy and physiology of central visual pathways,” in Adler’s Physiology of the Eye, edited by R. A. Moses (Mosby, St. Louis, 1975).

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Figures (6)

FIG. 1
FIG. 1

One of a series of computerized axial tomography scans shows the area of infarction in the inferior portion of the patient’s left cortex. The infarction extends to the posterior pole (three lower vertical arrows) and anteriorly into the temporal lobe (horizontal arrow). The small vertical arrow near the midline marks the ambient cistern which serves as a landmark confirming the inferior level of this scan.

FIG. 2
FIG. 2

Patient’s visual fields plotted with 0.92° test probe on a 32 apostilb background.

FIG. 3
FIG. 3

Patient’s color vision performance on two clinical tests. Upper graph illustrates his performance on the Farnsworth–Munsell 100 hue test. Note that the error scores were plotted on twice the conventional axis to accommodate the large score values. Lower graph plots his color matches (solid symbols) obtained on a model I Nagel anomaloscope.

FIG. 4
FIG. 4

Threshold measurements obtained in the patients’ parafovea. Left, test sensitivity for different wavelengths determined after 30 min of dark adaptation. The solid curve is the CIE scotopic luminous efficiency (plotted on a quantum basis) curve which was modified to include the intrusion of a long wavelength mechanism. The dashed curve is the patient’s foveal action spectrum (see Fig. 5). Right, increment threshold to a 500-nm test flash on a 500-nm steady field.

FIG. 5
FIG. 5

Threshold measurements in the patient’s fovea. Left, test sensitivity for different wavelengths determined after 10 min of dark adaptation. The smooth curve was drawn through the data points to highlight their overall trend. Right, increment threshold (solid triangles) to a 482-nm test flash on a red (Wratten No. 21) field. Open circles show the thresholds of a 68-year-old normal (deuteranopic) observer who was tested under similar conditions.

FIG. 6
FIG. 6

Patient’s foveal test sensitivity on intense chromatic backgrounds: (a) red (Wratten No. 21) background of 5.35 log td; (b) red (Wratten No. 21) background of 4.85 log td; (c) green (550-nm) background of 4.62 log td; and (d) blue (440-nm) background of 4.49 log td. For comparison, the data of the 68-year-old normal observer for condition (a) are also shown. The smooth curves (right) drawn through the data points are identical to that shown in Fig. 5 (left).